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What are the signs and symptoms of Cold agglutinin disease?

The blood of patients with cold agglutinin disease, when exposed to cold temperatures, behaves in ways that are quite different from the blood of healthy people. In affected individuals, certain proteins that normally attack bacteria (IgM antibodies), attach themselves to red blood cells and bind them together into clumps (agglutination). The antibodies activate other components of the blood, eventually causing red blood cells to be prematurely destroyed (hemolysis), thus causing anemia.[1]

Subsequent signs and symptoms can include weakness, dizziness, fatigue, headache, ringing in the ears (tinnitus), and spots before the eyes. Additional symptoms \may include irritability, bizarre behavior, absence of menstrual cycles in affected females (amenorrhea), gastrointestinal complaints, low levels of circulating red blood cells (anemia), enlargement of the spleen (splenomegaly), and/or persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice). In some cases, affected individuals may experience sweating and coldness of the fingers and/or toes, and uneven bluish or reddish discoloration of the skin of the fingers, toes, ankles, and wrists (called acrocyanosis or Raynaud's sign). Heart failure or shock may result in some cases.[1]

Cold agglutinin disease can be divided into primary or secondary forms. The signs and symptoms vary depending on type; but in general, symptoms are associated with the destruction of red blood cells, resulting in anemia. The primary form tends to be chronic and occur in older adults, ages 50 to 60. It can cause acrocyanosis or Raynaud's sign. Secondary forms tend to be acute, and resolve much more quickly. This form usually occurs in association with an underlying disorder such as infectious diseases, immunoproliferative diseases, or other autoimmune conditions.[2][3]

The Human Phenotype Ontology provides the following list of signs and symptoms for Cold agglutinin disease.
If the information is available, the table below includes how often the symptom is seen in people with this condition.
You can use the MedlinePlus Medical Dictionary to look up
the definitions for these medical terms.

Signs and Symptoms

Approximate number of patients (when available)

Arthralgia

90%

Autoimmunity

90%

Hemolytic anemia

90%

Muscle weakness

90%

Pallor

90%

Abnormality of urine homeostasis

7.5%

Diarrhea

7.5%

Hepatomegaly

7.5%

Lymphadenopathy

7.5%

Migraine

7.5%

Nausea and vomiting

7.5%

Splenomegaly

7.5%

Last updated: 3/1/2015

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.